Download Coarctation of Aorta Complicated by Bacterial

CLINICAL CONFERENCE
Editor: EDGAR V. ALLEN, M.D.
Associate Editor: RAYMOND D. PRUITT, M.D.
Coarctation of Aorta Complicated by Bacterial
Endocarditis and an Aneurysm of the Sinus of Valsalva
By FRANK GLENN, M.D., HAROLD J. STEWART, M.D., MARY A. ENGLE, M.D.,
DANIEL S. LUKAS, M.D., JOSEPH ARTUSmo, M.D., ISRAEL S. STEINBERG, M.D., AND
GEORGE R. HOLSWWADE, M.D.
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she began to improve and by the age of 9 years
she was average in size (fig. 1). She tired
after exercise and became short of breath
more quickly than her playmates. Restriction
of activities was advised by the physician. At
this time she had a murmur of aortic insufficiency. The first recorded blood pressure
determination was at age 12, measured at
165-145/65 mm. Hg in the arm.
In 1947 the patient had fever and blood
DR. FRANK GLENN: The patient for today 's
discussion presented sonme particularly
interesting and challenging problems when
she was first seen at The New York Hospital
in November 1954. I am going to ask our
panel to review with you the ideas and considerations that confronted us in the diagnosis
and management of this patient's heart disease. Dr. Engle, will you please give us the
history ?
DR. MARY A. ENGLE: The patient, a 27year-old married woman, was admitted for
evaluation of coarctation of the aorta. From
her and from the physicians who took care of
her from infancy we obtained the following
information. She was born 1 month prematurely. Because she failed to thrive, she was
taken to a pediatrician at the age of 51/2
months. He discovered a loud systolic murmur all over the precordium and in the lung
fields. The aortic and pulmonic second sounds
were distinct, and there was iio precordial
bulge. Fluoroscopy disclosed enlargement of
the left ventricle. These findings remained
unchanged throughout infanuv and childhood
and she was considered to have a ventricular
septal def ect.
Failure to thrive was most marked during
the first year. After the age of 2, however,
culture was positive for Streptococcus viridans. The blood pressure in the arms was
210/70 and in the legs 122/80 at this time.
She received a course of penicillin for 2 weeks.
She suffered a second episode of Str. viridans
infection in 1954. This was treated with intravenous penicillin and streptomycin for 6
weeks. The diagnosis of coarctation of the
aorta was made at this time. The cardiothoracic ratio at this time was 53.4 per cent.
DR. GLENN: Dr. Engle, is this the usual
course of events for a patient with coarctation
of the aorta?
DR. ENGLE: YeS, it is a common story. The
patient with coaretation of the aorta is apt
to have difficulty from his lesion at 2 age
periods: early infancy and early to mid-adult
life. Babies may fail to thrive and even go
into heart failure in the first months of life.
The early difficulties seem related to the
severity of the coaretation as well as to the
relationship of this constriction to the ductus
arteriosus, which is normally still patent in
the newborn period. Medical management of
the heart failure is quite successful in tiding
those babies over the period of early difficulty
From the Cardiovascular Conference Group made
up of members from the various departments of the
New York Hospital-Cornell Medical Center: Surgery,
Frank Glenn, George R. Holswade; Medicine, Harold
J. Stewart, Daniel S. Lukas; Pediatrics, Mary Allen
Engle, Henry P. Goldberg; Radiology, Israel Steinberg, Nathaniel Finby; Anesthesiology, Joseph Artulsio; Obstetrics, Curtis L. M-~ei(lelsOin.
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Circulation, Volume XVII, March 1958
COMPLICATED COARCTATION OF AORTA
AGE IN YEARS
AGE IN YEARS
0
150
40
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AGE IN YEARS
AGE IN YEARS
FIG. 1. Growth chart of patient V.W. Failure to grow was most marked during first year.
After age 2 it improved and by age 9 was in the average range. Blood pressure in arm at
(We are in12 years was 165-145/65 mlmi. Hg; at 16 years, 150/20; and at 18 years, 150/40.
(lebted to Drs. Benjamin Kramer and Charles Shookof for supplying the information on this patient 's childhood and adolescent years, including growth data.)
until adequate collateral circulation develops.
This is usually achieved by the age of 2 years.
This particular patient was not in heart
failure but she did have cardiac enlargement
at least from 5 months of age and her growth
was markedly retarded for the first year.
It is interesting that adults may not volunteer such a history even though this was a
difficult time in their lives. It is a period
beyond their memory, and unless a parent or
the physicians' notes are available, as in the
present case, this part of the history may be
lost.
DR. G-LENN: Dr. Stewart, if these childreix
survive childhood, in brief, what may be the
course of their disease?
DR. hAROLD J. STEWART: Certain patients
live a normal life span and die of some disease
not related to the congenital defect. On the
other hand during adulthood, patients with
coaretation of the aorta are subject to several
complications: (1) to subacute bacterial
endarteritis or endocarditis, which may occur
on the additional lesion of bicuspid aortic
valve, (2) to rupture of the aorta, (3) to a
cerebral vascular accident, (4) or to the manifestations of hypertensive cardiovascular disease, and perhaps heart failure, (5) heart
failure or rupture of the aorta, or of a
cerebral anieuirysmii, may result from the increased burden of pregnancy.
return to the case
DR. GLENN: Let
presentation. What were the pertinent findings on examination when this patient came
under observation here?
DR. ENGLE: Physical examination revealed
strong radial pulses and weak femoral pulses
with a perceptible lag behind the radial impulse. Blood pressure in the arms was
190/50-0 and in the legs 95/40 mm. of Hg.
A bounding pulsation was visible in the
sulprasternal notch, and pulsations could be
us
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FIG. 2. Roentgenograms. A. Frontal, showing enlargement of left ventricle, lack of aortic
knob prominence, anid rib notching. B. Lateral, showving anterior displacenient of esophagus
l)y enlarged ascending aorta.
seen and felt over the chest posteriorly. There
was no cyanosis or clubbing of fingers or toes.
The heart was enlarged to the anterior axillary line. A systolic thrill was palpable over
the aortic area and in the suprasternal notch.
A loud, long systolic murmur, maximal in
the second right interspace, was transmitted
to the neck vessels and the aorta, over the
precordium, and to the chest posteriorly. A
separate harsh systolic murmur was heard ill
the fourth and fifth left parasternal spaces.
A long, blowing diastolic murmur along the
left sternal border originated at Erb's point.
The lungs were clear, the liver and spleen
were not enlarged, there was no peripheral
edema, and embolic phenomena were not
detected.
Laboratory studies showed normal red
blood cell count, hemoglobin, and hematocrit
level, but the white blood cell count was 12,500
and the sedimentation rate was elevated.
Three blood cultures were negative.
X-ray Pnd fluoroscopy of the chest (fig. 2)
revealed a cardiothoracic ratio of 60 per cent.
The enlargement was predominantly left ventricular, though there was some enlargement
of the left atrium as well. The aortic knob was
normally prominent. In the left anterior
oblique projection the ascending aorta was
dilated anid widely pulsatile. Barium swallow
confirmed the left atrial enlargement anid
disclosed an anterior displacement of the
esophagn's by a dilated portion of the ascendincg aorta. This was thought (aand later
shown by angioeardiography) to be due to
the poststenotic dilatation of the aorta below
the coaretation. There were no other retroesop)hageal vessels, suggestive of aneurysmally
dilated intercostal arteries. Rib notehinig was
present bilaterally.
Anl electrocardiogram disclosed a pattern
of left ventricular hypertrophy (abnormally
tall R w-aves in the left precordial and foot
leads with deep S waves in the right precordial leads) and of left ventricular
"strain" (depression of S-T segments and
inversion of T waves in left precordial and
foot leads with reciprocal changes over the
right side of the precordium).
DR. GLENN: Dr. Lukas, will you give us
the pressure mneasuremernts you made?
DR. DANIEL S. LUKASS: Lntra-arterial pres-
COMPLICATED COARCTATION OF AORTA
tracings were characteristic of coaretation of the aorta and aortic insufficiency.
Measurements were 227/63 (mean 122) mnm.
Hg in the arm and 110/66 (inean 82) ill the
leg.
DR. GLENN: Dr. Stewart will you comment
on the diagnosis at this point?
DR. STEWART: Dr. Glenn, I think that the
diagnosis of coaretation of the aorta was
readily made clinically in this patient. The
volume of all the peripheral pulses should be
estimated clinically in every patient and the
diagnosis should be suspected when the volume of the pulses ini the legs is less than that
in the arms, or may indeed be absent. Moreover, the blood pressure should be taken not
only in both arms but also in both legs in
every patient in all age groups.
The following findings pointed to this
diagnosis: 1. The volume of the radial pulses
was greater than the femoral pulses. 2. The
blood pressure in the arms was higher than
in the legs, indicating, an obstruction to the
flow of blood through the aorta below the
subelaviati artery. It mliay be said parenthetically that the blood pressure may l)e normal
in the arms, but decreased to absent in the
legs. The carotid pulsations were exaggerated as were the aortic pulsations in the
suprasternal notch. 3. The peripheral signs
of collateral circulation shown by pulsations
and bruits over intercostal arteries posteriorly
and other collateral vessels used to carry
arterial blood from above the coaretation back
into the aorta below the obstruction. 4. The
x-ray of the chest showed notching or scallopingr of the lower margins of the ribs by the
dilated tortuous intercostal arteries. Both
the pulsations and notching may be absent in
children. The presence of these latter 2 signs
is a valuable guide to the surgeon as to the
possibility of a successful operation because
of the availability of these vessels to maintain
circulation while the aorta is clamped. The
x-ray showed left ventricular enlargement
and absence of the aortic knob. 5. The exact
location of the coaretation and the amount of
proximal segment beyond the subelavian
sure
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available to the surgeon for the repair was
demonstrated by angiocardiogram, which Dr.
Steinberg will discuss shortly.
Perhaps Dr. Engle has some additional
comment to make.
DR. ENGLE: Localization of the coarctation
was possible from the studies thus far presented. The fact that the pulses in the neck
and in both arms were equally strong placed
the obstruction distal to the left subelavian
artery. There was evidence on barium swallow of a large retroesophageal vessel below the
level of the descending aortic arch (fig. 2B).
Ill such a patient this deformity of the
esophogram is due to the poststenotic dilatation of the aorta just beneath the coarctation.
Thus the narrowed segment of aorta was
localized to the area of the aorta where it
most commonly occurs: at the level of the
ligamentnm arteriosum. One additional localizing sign that is useful is the smooth, blowing,
well-localized systolic murmur that is heard
posteriorly near the midline overlying the
narrowed segment. If one listens along the
course of the descending aorta, such a murmur can often be detected, even if the coaretation is in the abdominal aorta. This patient,
however, had such a loud systolic murmur
over her chest anteriorly and posteriorly that
this sign was less valuable.
Coaretation of the aorta is much less common in females than in males. When it is
present, it is apt to be in an unusual location
or complicated by some additional lesion. This
patient 's coaretation was in the usual location,
but the presence of an associated cardiac
lesion was suggested by the loud systolic murmur over the heart and chest that were
present from infancy. The murmur of a
coaretation itself is soft and is heard better
posteriorly over the descending aorta than it
is over the heart. With enlargement of the
left ventricle and dilatation of the mitral
valve ring or of the aorta, murmurs of relative
mitral insufficiency or aortic stenosis appear,
but her murmur was quite loud and unchanging from early infancy, even though her heart
was not mnch enlarged. A more likely ex-
43;3
CLINICAL CONFERENCE
LEFT COMMON CAROTID ARTERY
LEFT SUBCLAVIAN ARTERY
#NNOMINATE ARTERY
INTERNAL
A
WAMMARY
ARTERIAL
COLLATERALS
X-,~
-XX-
COARCTATION
(
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A
z
i.\A
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! WALL OF
LEFT
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SINUS OF
VALSALVA ANEURYSM
FIG. 3. Left anterior obliquie angiocardiograill 7 seconds after beginning the injection. Left.
N-ray Sloshwing hypertrophy of left ventricle, aneurysm of sinus of Valsalva, cOarCtaltioIn of aorta,,
and inuterna 1 mrnanima ry arterial collateral vessels. Righ t. Tracing of x-ray.
platation is that she had a congenital anomaly
of the aortic valve in the form of a bicuspid,
slightly stenotie aortic valve or subvalvular
ridge. Abnormalities of the region of the
aortic valve are commonly associated with
coaretation of the aorta.
DR. STEWART: In my experience the systolic
murmur in adults is heard best anteriorly
over the base of the heart. If it is very loud,
it may be heard posteriorly as well but it is
difficult to be certain whether a murmur heard
posteriorly may not be bruit over the collateral circulation.
The murmur of aortic insufficiency had
been heard in this patient at 12 years of age
before she had subacute bacterial endocarditis.
It is not uncommon to hear such a murmur
in these patients in the absence of any complication. I have thought that the murmur
was due to dilatation of the aortic ring by the
dilatation of her aorta with the hypertension
above the coarctation. Murmurs are not
heard in "normal" bicuspid aortic valves.
DR. GLENN: The history and findings given
by Dr. Engle justified the diagnosis of coaretation of the aorta, aortic regurgitation,
and bacterial endocarditis. Demonstration of
the exact location of the coarctation enables
the surgeon to plan his approach as well as to
be forewarned of additional possible abnormalities.
Dr. Steinberg, will you review the x-ray
findings including the angiocardiograms of
the patient ?
DR. ISRAEL STEINBERG: The conventional
roentgenograms showed left ventricular enlargement. There were absence of the aortic
knob and posterior indentation of the esophagus by a dilated descending aorta. Rib notehing was present bilaterally from the fourth
to seventh ribs. Angiocardiography showed
coaretation of the aorta at the usual site just
below the origin of the left subelavian artery
(fig. 3). There was dilatation of the ascending aorta and brachiocephalic arteries, and
large arterial collateral vessels were seen. A
COMPLICATED COARCTATION OF AORTA
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2.2-cm. saccular aneurysm of the right aortic
sinus was clearly visualized.
DR. GLENN: Dr. Steinberg, would you tell
us something about your experience with
aneurysms of the sinus of Valsalva?
DR. STEINBERG: The aortic sinuses are intracardiac and cannot be identified on conventional roentgenography. During angiocardiography they appear as dilatations at the root
of the aorta immediately above the aortic
valves and are best visualized in the left
anterior oblique view. Inconstant filling of
the coronary arteries during angiocardiography does not always allow individual
identification of the sinuses. However, in
the left anterior oblique view the right coronary sinus is regularly anterior to and just
behind the sternum. The aortic sinuses are
in close relation to all the cardiac chambers,
particularly the right atrium and ventricle.
The origins of the pulmonary artery, the interventricular septum, and the left atrium
are adjacent, whereas the superior vena cava
is more distant.
Aneurysms of the aortic sinuses (of Valsalva) are rare and are either congenital or
acquired. The acquired types are chiefly due
to syphilis- or bacterial endocarditis. The
congenital aneurysms are thought to be due
to a development defect in either the aorticopulmonary septum or the elastic tissue of the
aortic sinuses. Edwards and Burchell attribute congenital aneurysms to lack of continuity
between ,he aortic media and aortic ring. In
aortic sinus aneurysms the wall is made up of
the atrium instead of aortic media; pressure
within the aorta causes bulging of the sinus,
producing an aneurysm.
In an 11-year period, during which over
3,000 patients were studied by the intravenous
method of angiocardiography, 25 cases of unruptured aneurysms of the aortic sinus have
been diagnosed at this center. Ten were
acquired; 9 were due to syphilis and 1 was
due to arteriosclerotic dilatation of the
thoracic aorta. Fifteen were congenital and
of these 6, including the case herein presented,
were associated with coarctation of the aorta.
437
One patient had aneurysmal dilatation of the
aortic sinuses and pseudocoarctation of the
aorta while another had associated aortic and
mitral valvular disease. Seven patients had
arachnodactyly. Aneurysmal dilatation of
the aortic sinuses was found in all the cases
except the one reported here; in this, the
right aortic sinus alone was aneurysmal.
Aortic regurgitation, which is common in
patients with aortic sinus aneurysms, has been
attributed to dilatation of the aortic ring and
was present in all the acquired cases of aortic
sinus aneurysm. In congenital aortic sinus
aneurysms, anomalies of the aortic cusps have
been frequent at autopsy. In our series, in
addition to the case described above, aortic
incompetence occurred in a patient with
arachnlodactyly.
Congenital aortic sinus aneurysms are
paper thin and have a tendency to rupture
into the structures of the right side of the
heart. When perforation occurs, severe overloading of the right cardiac chambers, pulmonary hypertension, and intractable heart
failure follow. Sudden onset of dyspnea
associated with a machinery murmur suggests
rupture of the aortic root with creation of an
aorticocardiae fistula. Confirmation of this
event may be obtained by cardiac catheterization.
The incidence of aneurysm of the aortic
sinuses in coarctation of the aorta is difficult
to estimate; it occurred 6 times among 128
patients with coaretation of the aorta studied
angiocardiographically at this center. The
ages of patients varied from 13 to 27 years
with an average of 21 years. Five were males
and 3 females. Only 1 was a Negro. Three
patients were asymptomatic although a history of previous hypertension or heart murmurs was present in every instance. Headache
was present in 2, while 1 had bacterial endocarditis. Systolic murmurs (grade IIl to IV)
transmitted to the axilla and back, especially
over the suprascapula area and similar to
those usually found in coaretation of the
aorta, were common.
In all cases of aneurysmal dilatation of the
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aortic sinuses and coaretation there was enlargeinent of the heart, especially the left
ventricle. Deformity of the aortie arch and
rib notching were constant findings. Angiocardiography provided the definitive diagnosis
of aortie sinus aneurysm and demonstrated
the point of eoaretation. In the literature
there is also a report of a right aortic sinus
diagnosed by retrograde aortography.
DR. GLENN: Thank you. That is a beautiful demonstration.
Dr. Eiigle, will you tell us more of her
hospital course ?
DR. ENGLE: While these studies were being
performed, the patient ran a low-grade fever,
around 37.6 C., and had a persistently elevated white cell blood count and sedimentation rate. The possibility of incomplete cure
of the subacute bacterial endocarditis was
raised.
DR. GLENN: Dr. Stewart, what should be
done to evaluate such a situation? What
therapy would you recommend for bacterial
endocarditis ?
DR. STEWART: A persistent search should
be made for embolic manifestations: peripheral petechiae in nailbeds, fingertips, eyegrounds; repeated blood cultures at the height
of the fever. Arterial blood cultures have
not been more effective in securing positive
blood cultures than venous blood. The most
common organism that causes subacute bacterial endoearditis in this defect is the
Streptococcits virida ss. Should the blood
culture be positive, an optimal course of
therapy should be instituted.
It is the general experience that more relapses occur in patients treated for the shorter
periods and with the smaller dosage schedules.
Accordingly one starts out on a 6-week program of a total of 4 to 6 million units of
sodium penicillin G every day, given in
divided doses at 2-hour intervals intramuseularly. If later tests indicate that the strain
of the organism is a nonresistant one, the
amount of penicillin is increased. On occasion
the treatment may be stopped at 4 weeks.
If there is need for attempting to treat for
a shorter period, a rapid 2-week "combined "
CLINICAL CONFERENCE
therapy course may be used, namely, a total
of 6 million units of sodium penicillin G
intramuscularly in divided amounts every 2
hours, every day, and a total of 2 Gm. of
dihydrostreptomycin (or 1 Gm. of streptomycin and 1 Gm. of dihydrostreptomycini) in
4 doses intramuscularly as originally described by Robbiiis and Tompsett.
If repeated blood cultures are negative but
subacute bacterial endocarditis is still likely
or suspected, a course of therapy should be
given. Since 1 out of 5 instances of subacute
bacterial endocarditis with positive blood cultures is due to enterococcus, it is best to treat
those with negative cultures as though they
are of this worst type and give the combined
therapy for a period of 6 weeks.
DR. GLENNX: Dr. Stewart, will you pull together the diagnosis that finally emerged?
DR. STEWART: It was apparent that the
patient had coaretation of the aorta with very
high blood pressure above the coaretation, but
w-ith good collateral circ(llation. There was
the murmur of aortic insufficiency. The
angiocardiogranm showed the coarctation to be
located below the left subelavian artery, and
also denionstrated an aneurysm of the sinus
of Valsalva. The patient had had 2 episodes
of bacterial endocarditis or endarteritis, the
last one fairly recently. There was no w-ay
of ascertaining whether the sinus of Valsalva
wxas the site of the implantation, or perhaps
an associated bicuspid aortic valve, or the
coareted segment.
She was a patient with a complicated background who was presented for correction of
the coaretation of the aorta.
DR. GLENN: We anticipated 2 hazards in
particular with this patient. The first was
that an increase in the blood pressure following the application of occluding clamps prior
to resec tion of the coareted segment might
result in a rupture of the aneurysm of the
sinus of Vtralsalva. The second was the possible
requirement of prolonged occlusion of the
aorta because of technical difficulties that
might arise from the result of a long-standing
infection, the bacterial endocarditis.
Dr. Stewart, how soon is it safe to operate
COMTHPLICATED COARCTATION OF AORTA
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after recent subacute bacterial endocarditis?
DR. STEWART: Dr. Glenn, with this patient
with slight fever only, who had coaretation
of the aorta with Avery hi(rgh blood pressure
and an aneuryssm of the sinus of Valsalva,
the possibility of its rupture indicated that
repair of the coarctation, if it could be done,
should be carried out as soon as possible.
To this end she was given a 2-week course
of 1)enicillin therapy and was discharged
afebrile on DVcember 6. Because of nocturnal
cough, dyspiiea, and orthopniea as eviidelice of
early heart failure, the patient was digitalized. Shle was readmitted on February 13,
a)Jroximately 21/2 months later. This period
of time was allowed to permit healing of the
endocarditis and formation of strong scar
tissue in the aneurysm of the sinus of V'alsalv a
inl case this was involved.
DR. GLENN: Dr. Artusio, what problems
does this patient Present to tile anie.thesiologist ?
DR. JOSEPii ARTU SIO: We have used diethyl
ether in this clinic to accomplish a light level
of anesthesia, which we have termed analgesia.
The level is one in which there is minimal
depression to respiration and circulation, and
the patient frequently is oriented as to place
but is completely analgesic and has no memory of the experience. At this level of anesthesia the patient has optimal reactivity to
the stresses of surgery, blood loss, extreme
positions on the operating table, and change
of position on the operating table.
Premedication consisted of a small dose of
atropine, 0.2 mg., and in order that the circulation not be embarrassed by the presence of
an opiate, none was used. A 250-mg. dose of
thiopental sodium, in a 2.5 per cent solution,
was given for the psychic sedation afforded
the patient, so that she would not fear the
anesthetic mask. Nitrous oxide-oxygen was
started, with an 8-L. flow of 75 per cent nitrous
oxide and 25 per cent oxygen, and ether was
gradually added to the mixture until the patient entered a light level of surgical anesthesia. At that time, under direct vision, the
pharynx and larynx were sprayed with 2 per
cent Xylocaine for topical anesthesia, a 38-
439
French cuffed endotracheal tube was inserted
into the trachea, and the patient was immediately returned to the analgesic state of
anesthelsia where she responded to the spoken
voice.
Since this patient had an aneurysm of the
sinus of Valsalva in association with her coaretation of the aorta, we were most concerned
that during the period in which the proximal
aorta was clamped the blood pressure should
not rise unduly and produce rupture of the
aneurysm. It was planned to use Arfonad, a
thiophanium derivative, which would induce
ganglionic blockade in the periphery and
control the height of the blood pressure. llowever, from previous experience with this
(ompound we knew that the desired hypotension was not always achieved. This resistance
to hypotension is particularly true in the
youmigr adult group to which this girl belonged.
Thus it was considered that if Arfonlad did
not produce the necessary hypotension, eoncomitant hypothermia might aid to control
blood pressure.
Actually Arfonad worked very well and we
were able to reduce the blood pressure at will.
The blood pressure before the aortic clamps
were applied was 230/80 mm. Ilg with a
pulse rate of 80. Arfonad was started by
intermittent injections of 3 mg. followed by
a 0.1 per cent adjustable drip. In this manner the blood pressure was maintained within
a range of 140 to 200 systolic. Following the
anastomosis the blood pressure leveled off to
170/80.
DR. GLENN: Dr. Holswade, what are the
advantages of hypothermia in this situation
and how was it used?
DR. GEORGE R. HOLSWADE: It was hoped
that by cooling the patient to approximately 39 C. the blood pressure would fall
subsequent to a decrease in cardiac output
and would not rise precipitously during the
occlusion of the aorta. When surface cooling
of the body occurs, there is often a short
initial rise in blood pressure due in part to
peripheral vasoconstriction and possibly to
shivering in some cases. But as the cooling
progresses, the blood pressure falls in a curve
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440)
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that is parallel to that of the temlperatnre and
to that of the cardiac output. Cooling in a
patient such as this should not be carried to
a rectal temperature lower that 31 or 32 C.
In this temperature range there is little danger of serious cardiac arrhy thmias. This
degree of hypothermia might provide just
enough slowing of heart action, just enough
decrease in cardiac output and just enough
hypotensive effect to prevent, a rupture of the
aneurysm of the sinus of Valsalva when the
aorta is cross clamped. For this reason it
seemed advisable that the patient be cooled
while the chest was being opened and the
coaretation was being dissected free.
Cooling of the patient did not delay the
operation. It was easily accomplished by
pumping a cold alcohol solution through
special rubber blankets (Thermo-rite), which
were placed beneath the patient and over the
lower portion of the body. To further speed
up the cooling process plastic bags filled with
crushed ice were packed around the patient.
The temperature was reduced to 32 C. and
maintained at that level until the anastomosis
was completed. Warm water at 44 C. was
then pumped through the rubber blankets.
The patient remained on the operating
table until her body temperature had returned
to 35 C. She withstood the procedure extremely well and was awake at the end of
the operation.
DR. GLENN: The operation was accomplished with little more than the usual effort
required for an adult with a coaretation.
There was a very luxuriant collateral circulation. The intercostal arteries near their
origin from the aorta were almost aneurysmal.
Three pairs were divided. The internal mammary was very large.
The heart was enlarged, the left ventricle
being quite prominent, its wall hypertrophied.
The proximal aorta at its origin was enlarged
and a coarse thrill was felt in systole, but
perceptible in diastole. A saccular aneurysm
of the right aortic sinus measured 2.2 em. in
diameter, and projected 2 em. above the surrounding cardiac surface. It had an egg-shell
consistency and expanded very little on contraction of the left ventricle. Probably it was
B.
FIG. 4. Findings at operation. A. Coaretaltion
located 4 em. distal to stlubelavia.i. Aorta distal to
it enlarged, jet plaque palpable 2 emi. beyond on
the left lateral
asl)eCt
of
arterial
all.
B. Liga-
mentum a rteriosum entered coareted segmnent proximal to diaphragm. The lumien of the diaphragm
was slit-like and measured 2 mm. in its greatest
diameter. C. Wall of eoarle te(l segnuienit at level of
the diaphragm. X 12.
partially obliterated and, in the belief that it
might further decrease following the lowering
of the blood pressure after resection of the
coarctation, it was left unmolested. Furthermore, it has been demonstrated that the initracardiac approach seems to be the avenue of
preference.
The coareted segment measured 1 ce. in
length, and in its midportion as it was held
forward on slight tension, a diaphragm could
be palpated. A palpable thrill was quite
evident, with a jet that, could be felt most
distinctly 2 cm. beyond on the left lateral
aspect of the dilated distal segment. Here
there was a definite "jet plaque." The ligamenitum arteriosum entered the coareted segment just proximal to the diaphragm. It
measured 1 em. in length and 0.3 em. in
diameter. On palpation it seemed tubular and
possibly patent but there was no thrill. It
was divided, tiAe aortic half was patent but
441
COMPLICATED COARCTATION OF AORTA
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the 1l)ilniollary portion was obliterated (fig.
4A).
Clamps were placed proximal and distal to
the coaretation. There was an elevation of the
blood pressuire from 160/80 to 220/90 mm.
Hg. The coareted segmienit was reseeted, and
an aniastomliosis was done. This required almost 30 minutes and during this period
the 1loo0( pressure, althougrh fluctuating
betweeni 210/90 and 130/80, leveled off
at 170/80, being controlled with Arfonad.
After completion of the aniastomosis the distal
clamp was removed and then the proximal
(clam}a) was gra(nallyak released over an interval of 15 mniitecs. The blood pressure did
not fluetuate thereafter and remained at
170/80.
The lnmeii of the diaphragm ini the resected
semeint was slit-like in shape andl measured
2 mni. ini its greatest (liameter. There were
numerous; liniear bright yellow fibronis plaques
in the diaphragm aln( the adjacent wall of
the aorta (fig. 4B).
The wa'all of the aorta was thickened in the
area of (coar(atioll and its pattern was somewhat disrlu)ted (fig. 4C). At the jinetion of
the thickened poortion and the more normal
vas(enlar w-all there was ani atheromatous area
conisistingt of a colleetion of large mononuclear plhago(ytes with foamy- cytolasm. Connectiv\e tisslle was abundant ini the wall of the
aorta an(1 ini the area of diaphragmatic narrowing of the coaretation the usual parallel
pattern hlad disappeared and the fibers were
tangrled, twisted, and turned npoIi one another.
Her immediate postoperative course was
unevenitfnil and without complications. She
was discharged 3 weeks later. At that time
the blood pressures were right arm 150/55;
left arm, 160/60; right leg, 210/110; left leg,
200/100.
Dr. Engle, what has happened to this patient since she left the hospital?
DR. ENGLE: After convaleseenc( e she returnied to her regular occupation as a freelance writer. It is now 21/2 years since the patienit 's operation. She states that she has never been so energetic or untiring. She can
climb 3 or 4 flights of stairs before her legs
fatigue. She does not experience shortness of
FIG. 5. wOPcntgenlogralil 30 months after resect ion
of eo .llreta.tioi showving decrease in size of left v eii~
tricle.
breath, aiid she sleeps flat comfortably. She no
longer takes a digitalis preparation.
Her blood pressure is 130/50 - 30 mmn. Hg
in the arms and 140/50 ill the legs. Exaggerated intercostal pulsations are gone. The
aortic systolic mnurmur and the diastolic murmur of the aortic inisuffieiency persist. The
aortic pulsation in the suprasternal notch is
still evident. Tlie heart size is less by 1 em.
than preoperatively, and this improvement is
due chiefly to a decrease ill left ventricular
size (figy. 5). There is Iio loniger evidence of
left atrial enlargement, and the ascending
aorta is smaller. 11er electrocardiogram continues to show left ventricular hypertrophy
and "strain."
Thus the manifestationis of (oaretation of
the aorta have disappeared. Relief of this
cardiac. burden has apparently made her better able to withstand the aortic valve involvement. Objectively, as well as symptomatically,
she is better. She will continue under medical
supervision.
DR. STEWART: Dr. Glenn, what has been
our experience with aortic insufficiency in coaretation of the aorta?
DR. GLENN: Aortic insufficienicy is seeni ill
coaretation under 3 circumstances. The first
is believed to be due to prolonged hyperten-
CLINICAL CONFERENCE
442
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sion that produces enlargement of the proximal aorta and the aortic ring at the level of
the valves. Secondly the aortic valve may
have only 2 leaflets instead of 3, and slight
enlargement of the aorta may result in insufficiency. Then in the third group there are
those instances where disease such as bacterial endocarditis or rheumatic heart disease
may cause a loss of valve substance that results in insufficiency. Our experience with
aortic insufficiency associated with coaretation of the aorta is limited. We have operated
upon 4 such patients. All 4 tolerated the operation well. With a return of the blood pressure to normal, 1 patient lost the signs of insufficiency. Another, although experiencing
relief from the preoperative hypertension,
continued to have signs of aortic insufficiency
and increasing left heart failure, and died
almost a year later. The other 2 patients have
had a persisteuee of definite evidenc(e of nor-
tie regurgitation l)ut are definitely improved.
This patient, our fifth with aortic insufficiency, continues to have a systolic and diastolic
murmur along the left sternal border. Evidently she has a definite aortic regurgitation,
now 30 months after operation.
DR. STEXVART: Durinog the course of the 30
years of this woman 's life, advances in medicine and surgery have come along just in
time to help her out of difficulty: antibiotic
therapy for subacute bacterial endocarditis
and surgery for removal of the coaretation.
If she should later need help because of aortic insufficiency or rupture of anl aneurysm
of the sinus of Valsalva, these conditions too
are now within the ever-widening area of
cardiovascular lesions, both congenital and acquired, that are aimieniable to surgery. Close
teamwork of the medical and surgical specialties interested in this field has made possible
these great steps forward.
9
Welch, J. S., Kirklin, J. W., Ellis, F. H., Jr., and Bruwer, A. J.: Resection and Grafting for Chronic Occlusion of the Terminal Aorta or Iliac Arteries. J.A.M.A. 164:
1045 (July 6), 1957.
The recent Mayo Clinic experience in the management of aortic and iliac arterial occlusive disease is described. In the present series of 99 patients, intermittent claudication
was the chief symptom and the status of the leg pulses was the most important physical
finding. Preoperative aortography was done routinely in order to define the extent of
the occlusions. Technical aspects of resection of the obstructed vessels and homograft
reconstruction are outlined. Excellent operative results were obtained in 71 patients
(72 per cent) as indicated by complete relief of symptoms and return of leg pulses. Five
deaths occurred, all postoperatively. The most important factor in the success of this
surgery is insuring that the distil alnastomosis is made to a patent artery, if necessary
to the superficial femoral.
ROGERS
Coarctation of Aorta Complicated by Bacterial Endocarditis and an Aneurysm
of the Sinus of Valsalva
FRANK GLENN, HAROLD J. STEWART, MARY A. ENGLE, DANIEL S.
LUKAS, JOSEPH ARTUSIO, ISRAEL S. STEINBERG and GEORGE R.
HOLSWADE
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Circulation. 1958;17:432-442
doi: 10.1161/01.CIR.17.3.432
Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX
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Copyright © 1958 American Heart Association, Inc. All rights reserved.
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